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Kaplan Qbank USMLE



Author6 Posts
  #1

A four-year-old white female presents to the pediatric clinic for follow-up due to developmental delay. Her medical history reports frequent episodes of otitis media, bronchitis, and pneumonia. Physical examination reveals coarse facial features, gingival hypertrophy, joint stiffness, claw hand deformity, and generalized hypotonia. Significant motor delay is appreciated. Electron microscopy of muscle biopsy demonstrates numerous intracytoplasmic inclusions, which are membrane-bound vacuoles filled with fibrillogranular material. I-cell disease is suspected. What is the function of the deficient enzyme?

A. Breakdown of dermatan sulfate and heparan sulfate

B. Cleavage of disaccharides

C. Cleavage of fructose

D. Degradation of sphingolipids

E. Metabolism of galactose

F. Phosphorylation of mannose residues


D?
there are accumulation of mucolipids, mucopolysaccarides, glycolipids, sphingolipids...

  #2

answer-f---to bring product to the lysosome instead of cytoplasm

___________________
Smell the coffee! "Is That an Osler move??"

  #3

F for sure

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The Key to Succeed is Patience.

  #4

Sialidosis
found it on e-medicine,and let me tell I am

shocked Choice E

  #5

Choice F

sorry it is late

  #6

F...







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